Ebstein's Anomaly

Surgery

The type of surgical repair depends on the size of the right ventricle and condition of the valve leaflets. The timing of surgery is very important. Artificial and, to a lesser extent, repaired valves may not last a lifetime and further surgery may be needed. The fewer operations required over a lifetime, the less chance for complications. Surgery may not be appropriate for all patients with Ebstein's anomaly. Rarely severe Ebstein's anomaly with severe valve involvement and reduced heart function is treated with heart transplantation.

Because Ebstein's anomaly is quite rare, it is very important to undergo surgery at a medical institution familiar with the defect, and the surgeon should be experienced with the procedure.

Repair of the tricuspid valve
This generally involves creation of a competent valve by reducing the size of the valve opening and allowing the existing valve leaflets to come together. This is done if there is enough valve tissue to allow for repair, it is not possible in all cases of Ebstein's anomaly.

Replacement of the tricuspid valve
The valve is replaced by removing the deformed valve and inserting either a mechanical valve or a specially treated tissue valve. If a mechanical valve is used, lifelong use of a blood thinning medication is required.

Closure of the Atrial Septal Defect (ASD)
Closure of ASD is done to prevent venous (unoxygenated) blood, which should be going to the lungs, from mixing with arterial (oxygenated blood coming from the lungs and going to the body) circulation.

Maze procedure
A right-sided Maze operation sometimes is appropriate. During the operation, the surgeon creates a series of incisions in the right atrium to interrupt the electrical pathways. Both the Maze procedure and the surgical interruption of an extra electrical pathway are usually done at the time of tricuspid valve repair or replacement.